MPS VI associated ocular phenotypes in an MPS VI murine model and the therapeutic effects of odiparcil treatment.

Maroteaux - Lamy syndrome (mucopolysaccharidosis type VI, MPS VI) is a lysosomal storage disease resulting from insufficient enzymatic activity for degradation of the specific glycosaminoglycans (GAG) chondroitin sulphate (CS) and dermatan sulphate (DS). Among the most pronounced MPS VI clinical manifestations caused by cellular accumulation of excess CS and DS are eye disorders, in particular those that affect the cornea. Ocular manifestations are not treated by the current standard of care, enzyme replacement therapy (ERT), leaving patients with a significant unmet need. Using in vitro and in vivo models, we previously demonstrated the potential of the ß-D-xyloside, odiparcil, as an oral GAG clearance therapy for MPS VI. Here, we characterized the eye phenotypes in MPS VI arylsulfatase B deficient mice (Arsb-) and studied the effects of odiparcil treatment in early and established disease models. Severe levels of opacification and GAG accumulation were detected in the eyes of MPS VI Arsb- mice. Histological examination of MPS VI Arsb- eyes showed an aggregate of corneal phenotypes, including reduction in the corneal epithelium thickness and number of epithelial cell layers, and morphological malformations in the stroma. In addition, colloidal iron staining showed specifically GAG accumulation in the cornea. Orally administered odiparcil markedly reduced GAG accumulation in the eyes of MPS VI Arsb- mice in both disease models and restored the corneal morphology (epithelial layers and stromal structure). In the early disease model of MPS VI, odiparcil partially reduced corneal opacity area, but did not affect opacity area in the established model. Analysis of GAG types accumulating in the MPS VI Arsb- eyes demonstrated major contribution of DS and CS, with some increase in heparan sulphate (HS) as well and all were reduced with odiparcil treatment. Taken together, we further reveal the potential of odiparcil to be an effective therapy for eye phenotypes associated with MPS VI disease.

Patient-to-patient transmission of hepatitis C virus through the use of multidose vials during general anesthesia.

A cluster of four patients with hepatitis C virus (HCV) infection was identified in a surgery clinic. Molecular characterization revealed close homology between viruses. This cluster was related to unsafe injection practices through multidose vials and reused materials. Among 796 patients potentially exposed to and screened for HCV, no other cluster was identified.

Nosocomial urinary tract infections in urologic patients: assessment of a prospective surveillance program including 10,000 patients.

OBJECTIVE: Hospital-acquired urinary tract infections (HUTI) represent a significant impairment in the quality of health care. Incidence in catheterized patients has been estimated at approximately 20%, however few data are available in urologic patients. We report a prospective surveillance program over 6 years in our urologic department and evaluate its evolution. METHODS: Population consists of all patients admitted to the urology ward for 48 hours or more over a 6-year period from 1994. Data recorded: age, gender, duration of stay, insertion and removal of catheters, diagnosis of HUTI. ANALYSIS: calculation of incidence, and incidence density for HUTI and for catheter-related HUTI, analysis of trends by chi(2) trend test. RESULTS: A total of 10,054 consecutive patients were included, 52% were catheterized. The median incidence of catheter-related HUTI in catheterized patients was 13.0%, the incidence density was 25.1 HUTI/1000 patient-days of catheterization. The proportion of HUTI and specific catheter-related HUTI patients decreased, respectively from 8.4% and 14.2% to 6.5% and 12.3% during the study period (p<0.05). CONCLUSION: The rate of HUTI was not as high as previously reported, perhaps due to a controlled catheter policy. Surveillance was associated with a significant decrease in infection rates, suggesting a beneficial feedback effect. Evaluation of diagnoses and surgical procedures would ensure an optimal quality control program.